Table 3.

Proportion of COX negative fibers, RBFs and intermediate fibers.

Case	Genetic defect	%COX negative (including RBF’s)	%COX intermediate
P1	OPA1	2.53	2
P 2	OPA1	5	4.3
P 3	POLG	28.3	20.7
P 4	POLG	12.5	16
P 5	SINGLE DELETION	28.5	16.5
P 6	SINGLE DELETION	3.94	18.5
P 7	SINGLE DELETION	10.8	20